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Objective Magnetic resonance imaging and computed tomography provide good anatomic detail of suprasellar tumors in pediatric patients but are not able to predict histology in many cases. Proton magnetic resonance spectroscopy provides metabolic data that may add to diagnostic specificity. We preoperatively performed localized proton magnetic resonance spectroscopy on pediatric patients with suprasellar tumors and correlated the results with the histological findings. Cyst fluid obtained from patients with craniopharyngiomas was studied with high-resolution magnetic resonance spectroscopy to better understand the in vivo data. Methods Nineteen patients aged 1 to 21 years underwent spectroscopy. Surgical pathological samples were obtained from 14 patients. In each of five patients, the presence of a solid chiasmatic mass in addition to clinical evidence of neurofibromatosis Type I allowed the presumptive diagnosis of chiasmatic astrocytoma. Thus, the study population included 6 patients with craniopharyngiomas, 10 with chiasmatic/hypothalamic astrocytomas, and 3 with pituitary adenomas. The data obtained were compared with those of healthy brain from age-matched participants. Results Spectroscopy was specific for the diagnosis. All craniopharyngiomas showed a dominant peak at 1 to 2 ppm, consistent with lactate or lipids, with trace amounts of other metabolites. This was confirmed using high-resolution spectroscopy. Chiasmatic gliomas showed a profile of choline, N-acetylaspartate, and creatine, and the choline:N-acetylaspartate ratio was 2.6 +/- 1.3, compared with 0.7 +/- 0.3 for samples of healthy brain (t test, P = 0.0003). Pituitary adenomas showed only a choline peak or no metabolites at all. Conclusion Proton spectroscopy may be helpful in supplementing standard imaging for the preoperative diagnosis of three types of suprasellar tumors that are common in pediatric patients.

Gangliogliomas account for 1-4% of all pediatric CNS tumors. We reviewed the records of 123 patients treated at the Children's Hospital of Philadelphia between 1974 and 1995. Ninety-nine patients were eligible for review with complete medical records. The mean age was 9.5 years with 52% females. The mean follow-up was 3.7 years. The most common presenting symptom was seizures (49%). The mean length of symptoms to diagnosis was 24.4 months. Complex partial seizure was the most frequent seizure type (60%). Electroencephalograms were abnormal in 36%. The majority of gangliogliomas were in the temporal lobes (38%), with other sites in the parietal (30%) and frontal lobes (18%). All patients had abnormal nonspecific neuroimaging studies. Nine received chemotherapy, and 21 were treated with radiotherapy. Postoperatively, 78% were seizure free on no medication, 18% had improvement of seizure control, and 4% had stable seizure events. Nine died of disease. Gangliogliomas are benign tumors that frequently present with seizure disorders and nonspecific electroencephalographic and neuroimaging studies. Complete surgical extirpation of these tumors provides improved seizure control in most patients and a short course of antiepileptic medical therapy. Adjuvant therapy including radiotherapy and chemotherapy is limited to recurrent disease not amenable to reoperation.

We report three patients with gangliogliomas involving the optic chiasm via distinct mechanisms.The ganglioglioma in one patient likely originated in the temporal lobe and spread medially to involve the chiasm, and diffuse spinal cord dissemination also occurred. Chiasmal involvement in this manner and dissemination at presentation are unusual for gangliogliomas. The tumor in a second patient was intrinsic to the hypothalmus and chiasm, while in the third patient, it involved both optic tracts, and a cyst compressed the chiasm laterally. Two patients developed severe bilateral visual loss, while the other had a stable bitemporal hemianopsia. Two patients received radiotherapy, but one continued to lose vision. Although gangliogliomas rarely involve chiasm, the mechanisms by which they produce chiasmal visual loss may be diverse, and the long-term visual prognosis is variable.NEUROLOGY 1996;46: 1669-1673

✓ The feasibility of radical surgery for astrocytomas of the optic chiasm/hypothalamus has been reported by several groups. Such surgery carries significant risks, however, including permanent damage to the pituitary gland, optic apparatus, hypothalamic structures, and carotid arteries. The benefits of radical surgery, both in terms of efficacy and toxicity, should, therefore, be evaluated against standard therapy, as is usually done for new chemotherapeutic protocols. To this end, a retrospective review was performed of 33 patients treated at Children's Hospital of Philadelphia between 1976 and 1991 who met criteria that would have made them eligible for radical surgery in many centers today, but were treated with either no surgery or conservative surgery (< 50% resection) or biopsy followed by adjuvant therapy with local radiation therapy (29 patients) and/or chemotherapy with actinomycin-D and vincristine (18 patients). The review encompassed all children with a globular enhancing mass of at least 2 cm in the hypothalamic/chiasmatic region, no evidence of optic nerve involvement or involvement of the optic radiations by computerized tomography or magnetic resonance imaging, and follow up of at least 3 years. All but one patient had tissue confirmation of a low-grade or pilocytic astrocytoma. Thirteen of the patients were 2 years of age or younger at diagnosis. Five individuals died: three of tumor progression, one of acute shunt malfunction, and one of intercurrent infection. The remaining 28 were alive at last follow up, a mean of 10.9 years from diagnosis. Twenty-three surviving patients have functional vision in at least one eye, 12 require no endocrine replacement, and 16 are in or have completed schooling with regular academic requirements. If radical surgery is to become standard care for children with low-grade astrocytomas of the hypothalamic/chiasmatic region, long-term survival and functional outcome will have to equal or surpass those of historical controls who were treated conservatively.

Background. Focal spasticity of the gastrocnemius-soleus muscles causes equinus gait in children with cerebral palsy (CP). Botulinum toxin type A (BTX-A), a neuromuscular blocking agent, reduces muscle tone/overactivity in dystonia, stroke, and CP. Objective. A prospective, open-label, multicenter clinical trial evaluated the long-term safety and efficacy of repeated intramuscular injections of BTX-A on equinus gait in CP children. Methods. Nine centers enrolled 207 children. BTX-A injections (4 U/Kg) were given approximately every 3 months (maximum dose 200 U per treatment). Outcome measures included a Physician Rating Scale of gait, ankle range of motion measurements, and the incidence and profile of adverse events. Results. One hundred fifty-five (75%) of 207 children completed at least 1 year with a total of 302 patient years of BTX-A treatment. The mean duration of BTX-A exposure was 1.46 years per patient. Dynamic gait pattern on the Physician Rating Scale improved in 46% of patients (86/185) at first follow-up. The response was maintained in 41% to 58% of patients for 2 years. Both gait pattern and ankle position improved at every visit. The most common treatment-related adverse events included increased stumbling, leg cramps, leg weakness, and calf atrophy in 1% to 11% of patients. No treatment-related serious adverse events were reported. Only 6% (7/117) of patients with pre- and postantibody samples had both detectable antibodies and a subsequent treatment failure. Conclusion. BTX-A proved both safe and effective in the chronic management of focal muscle spasticity in children with equinus gait.

Objectives: To estimate the rate of progression of plexiform neurofibroma after surgery and to identify prognostic factors that predict progression. Study design: A retrospective review of the inpatient and outpatient records of 121 patients, who had 302 procedures on 168 tumors over a 20-year period at a single large pediatric referral center. Data on age, location, indication for surgery, and extent of resection was analyzed for prognostic significance. Results: The overall freedom from progression was 54%. Children < 10 years old had a shorter interval of tumor control than older children ( p = 0.0004). Tumors of the head/neck/face fared worse than tumors of the extremities ( p = 0.0003). Less extensive resection predicted shorter interval to progression ( p < 0.0001). Indication for surgery was not of prognostic importance. In multivariable analysis older age and location in the extremities were predictors of a better outcome. Conclusions: Tumor progression is a serious problem for children with plexiform neurofibroma. Younger children, children with tumors of the head/neck/face, and tumors that cannot be nearly completely removed are at particular risk. These data may be useful in helping clinicians decide which patients and which tumors are most likely to benefit from surgical intervention. (J Pediatr 1997;131:678-82)

We describe the clinical and imaging findings of brain stem tumours in patients with neurofibromatosis type 1 (NF1). The NF1 patients imaged between January 1984 and January 1996 were reviewed and 25 patients were identified with a brain stem tumour. Clinical, radiographical and pathological results were obtained by review of records and images. Brain stem tumour identification occurred much later than the clinical diagnosis of NF1. Medullary enlargement was most frequent (68 %), followed by pontine (52 %) and midbrain enlargement (44 %). Patients were further subdivided into those with diffuse (12 patients) and those with focal (13 patients) tumours. Treatment for hydrocephalus was required in 67 % of the first group and only 15 % of the second group. Surgery was performed in four patients and revealed fibrillary astrocytomas, one of which progressed to an anaplastic astrocytoma. In 40 % of patients both brain stem and optic pathway tumours were present. The biological behaviour of brain stem tumours in NF1 is unknown. Diffuse tumours in the patients with NF1 appear to have a much more favourable prognosis than patients with similar tumours without neurofibromatosis type 1.

OBJECTIVE We have evaluated the frequency of endocrine abnormalities in a large group of patients with hypothalamic/chiasmatic glioma (H/CG) and its correlation with the different forms of therapy. DESIGN Descriptive retrospective study using case note review analysis. PATIENTS The records of 68 children who survived H/CG were analysed. One third had neurofibromatosis. The mean age at tumour presentation was 5 years. The median time of follow-up was 3.6 years. Thirty-eight children received cranial radiation, of whom 17 also had surgery. Surgery was performed in a total of 24 patients. Fifteen patients received only chemotherapy. Eight children, all with neurofibromatosis, received no specific tumour treatment. MEASUREMENTS Endocrine dysfunction was determined by clinical manifestations and biochemical evaluation of hypothalamic-pituitary function. RESULTS Endocrine dysfunction occurred in 42% of the children. The most common disorder was GH deficiency (GHD). Of 50 children evaluated, 15 of the 19 with GHD received cranial irradiation (P

The natural history and the clinical and neuroimaging features of brainstem tumors in neurofibromatosis type 1 (NF1) are poorly understood.Magnetic resonance imaging (MRI) has been useful in NF1 in detecting intracranial abnormalities, especially of the brainstem. Brainstem tumors in NF1 have been confused clinically with non-NF1 brainstem tumors and radiographically with the increased T2 signal abnormalities, also known as "unidentified bright objects" (UBOs), which are common in NF1 and often located in the brainstem. This study, which evaluated 17 NF1 patients with brainstem tumors, is the largest series to date. Fifteen of 17 patients (88%) had neurologic signs and symptoms referable to brainstem dysfunction, including dysarthria, cranial neuropathies, and gross motor incoordination. Tumors were located primarily in the medulla in 14 of 17 NF1 patients (82%), in contrast to the pontine tumor location in the non-NF1 population. Seven NF1 patients (41%) required shunt placement for hydrocephalus at initial diagnosis, more frequent than in non-NF1 brainstem tumor patients. Six of 17 patients (35%) had evidence of radiographic tumor progression, but only three of them (18%) had correlative clinical progression. Two patients with progressive symptoms had partial surgical resection, and pathology revealed either fibrillary or anaplastic astrocytomas. Three patients were treated with radiation therapy, chemotherapy, or both, with two deaths. With a median follow-up of 52 months, 15 of 17 patients remain alive; 14 of them did not require adjuvant therapy. In our series, we describe NF1 brainstem tumors as a distinct clinical entity, much less aggressive than non-NF1 pontine tumors but more symptomatic than brainstem UBOs in NF1. NEUROLOGY 1995;45: 1897-1902

Abnormalities of embryogenesis and nervous system development may cause or contribute to the development of childhood brain tumors. To identify genetic or environmental factors that may be associated with etiologies of childhood central nervous system tumors, we examined family histories of 165 children with such tumors for the presence of neurologic disorders, including neural tube defects, mental retardation, seizures, and central nervous system tumors, as well as other cancers and birth defects. Only 1 patient, with the neurofibromatosis-Noonan syndrome, was confirmed to have an underlying syndromic diagnosis associated with central nervous system tumorigenesis. Families of 2 probands with posterior fossa primitive neuroectodermal tumors reported relatives with olivopontocerebellar atrophy. Although increased incidences of study disorders were not identified in this population, it is possible that within individual families one or more of these disorders is related to childhood central nervous system tumorigenesis.